Lamotrigine-Associated Hemophagocytic Lymphohistiocytosis

Ignaszewski, Maya; Ignaszewski, Martha J.; Kohlitz, Patrick

doi:10.1097/mjt.0000000000000515

Cited by 13 publications

(18 citation statements)

References 2 publications

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“…Importantly, this patient made a complete recovery after being treated with dexamethasone/etoposide induction therapy and has not required maintenance therapy. Although our patient had several favorable clinical factors, such as no HLH-associated germline mutations, age under 50 years, no organ dysfunction at baseline, and a short time to treatment [2], her favorable outcome and that of a prior report [11] suggest that lamotrigine-induced HLH may have a better long-term prognosis relative to other secondary forms of HLH, provided the patient is not re-exposed to the offending agent.…”

Section: Discussionmentioning

confidence: 98%

“…Recently, the US Food and Drug Administration (FDA) release a safety announcement in April 2018 to warn that lamotrigine can cause a rare but severe immune system reaction meeting diagnostic criteria for HLH [10]. Eight known cases had been identified by the time of this release; we have now presented a ninth [10, 11]. HLH is characterized by uncontrolled inflammation and immune system activation often with devastating consequences.…”

Section: Discussionmentioning

confidence: 99%

“…While medications have been previously linked to secondary HLH, drug-induced HLH is less common than other causative etiologies [6, 7] and importantly there is no mention of the inclusion of such patients in the HLH-94 trial which established the standard-of-care treatment for this disease [8]. We suspect the underlying pathophysiology relates to a drug hypersensitivity reaction triggering an excessive activation of macrophages with the resultant hyperinflammatory sequelae; however, with only one prior case report of lamotrigine-induced HLH [11] data are lacking to identify more specific mechanisms. Importantly, this patient made a complete recovery after being treated with dexamethasone/etoposide induction therapy and has not required maintenance therapy.…”

Section: Discussionmentioning

confidence: 99%

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Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report

2019

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BackgroundHemophagocytic lymphohistiocytosis is a rare hematological syndrome characterized by excessive and uncontrolled activation of the immune system. The often nonspecific nature of early symptoms and the potential for progression to multiorgan failure and death if appropriate therapy is not started promptly, highlight the importance of heightened recognition for this uncommon disease. Although there are well-described associations of hemophagocytic lymphohistiocytosis with infectious, malignant, and autoimmune diseases and an established treatment protocol for these cases, the link between medications and hemophagocytic lymphohistiocytosis is less clearly established and the optimal treatment of these cases less well defined.Case presentationHere we describe the case of a 45-year-old caucasian woman presenting with signs and symptoms consistent with hemophagocytic lymphohistiocytosis, induced by recent exposure to lamotrigine. She had a rapidly progressive clinical course, complicated by multiorgan failure including stress-induced Takotsubo cardiomyopathy and cardiac arrest. With dexamethasone and etoposide therapy, she made a full and sustained recovery.ConclusionsThis case highlights that medication-induced hemophagocytic lymphohistiocytosis appears to respond similarly to the same dexamethasone and etoposide treatment regimen developed for other non-drug-induced forms of hemophagocytic lymphohistiocytosis. With the continued cessation of the offending agent there has not been need for maintenance therapy and no relapse to date. In addition, given the risk for cardiomyopathy, a clinical complication not classically associated with hemophagocytic lymphohistiocytosis, echocardiogram and telemetry monitoring should be considered in the initial workup of suspected hemophagocytic lymphohistiocytosis.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report

2019

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“…Drug‐associated HPS/HLH remains very rare, although it was very recently reported that antiepileptic drugs could cause drug‐associated HPS/HLH 7 . In addition, it has been reported that in some cases, drug‐associated HPS/HLH occurs as a result of systemic inflammatory response, such as drug rash with eosinophilia and systemic symptoms, and as a result of secondary infection due to immunosuppressive drugs in others; thus, in most cases, the syndrome is caused indirectly.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis with advanced malignant melanoma accompanied by ipilimumab and nivolumab: A case report and literature review

Mizuta¹,

Nakano²,

Takahashi³

et al. 2020

Dermatologic Therapy

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Combination therapy with nivolumab + ipilimumab was recently approved for treating unresectable cases of malignant melanoma. In spite of the high response rate, it is associated with a high incidence of serious adverse events, including immune‐related hemophagocytic syndrome/hemophagocytic lymphohistiocytosis (irHPS/HLH), a difficult to diagnose rare disease. This is the first report of this disease in an Asian malignant melanoma patient treated with nivolumab + ipilimumab. A 69‐year‐old Japanese woman with unresectable malignant melanoma was treated with nivolumab + ipilimumab. Following the combined therapy, her fever and symptoms of malaise occurred, and she visited to our hospital's emergency department. Blood tests revealed significant liver dysfunction, anemia, and thrombocytopenia. We suspected irHPS/HLH, based on tests revealing decreased fibrinogen and significantly increased ferritin. Bone marrow biopsy revealed numerous macrophages and high hemophagocytosis levels. After 50 mg prednisolone (1 mg/kg per day) was administered, fever and cytopenia markedly improved. irHPS/HLH has a high rate of coagulation abnormalities accompanied by hypertriglyceridemia and hypofibrinogenemia, which are unlikely to occur in adult HPS/HLHs. Because irHPS/HLH responds better to steroids than other secondary HPS/HLHs, we expect a complete cure with steroids. Quick diagnosis and appropriate treatment based on clinical symptoms and laboratory tests are needed in suspected cases.

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“…Treatment emphasizes on controlling the underlying cause and administrating immunosuppressive, immunomodulatory and cytotoscie medications. The disease has a high mortality if left untreated, and for this reason, immediate diagnosis and initiation of treatment are essential to boost the chances of survival in affected patients [3].…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis associated with Epstein–Barr virus infection: case report and literature review

Bahabri

Rikabi

Alshammari

et al. 2019

Journal of Surgical Case Reports

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Hemophagocytic lymphohistiocytosis (HLH) is rare and life threatening syndrome. There are only a few reported cases of HLH with GI symptoms. We describe the case of an 18 months old boy who presented with a history of fever for 40 days, abdominal distention and hepatosplenomegaly. Abdominal x-ray showed a pneumoperitoneum. Urgent laparotomy was done which revealed an isolated cecal perforation. The histopathological findings in the subsequent resected bowel was HLH with evidence of positive EBV Barr infection.

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Lamotrigine-Associated Hemophagocytic Lymphohistiocytosis

Cited by 13 publications

References 2 publications

Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report

Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report

Hemophagocytic lymphohistiocytosis with advanced malignant melanoma accompanied by ipilimumab and nivolumab: A case report and literature review

Hemophagocytic lymphohistiocytosis associated with Epstein–Barr virus infection: case report and literature review

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